Autopsy and Case Reports
Autopsy and Case Reports
Autopsy Case Report

Intravascular large B-cell lymphoma presenting with fever and refractory acidosis

Michael Harrison Storandt; Mark Alan Koponen

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Intravascular large B-cell lymphoma (IVLBCL) is a rare form of diffuse large B-cell lymphoma, characterized by malignant B-cells primarily localized to the lumina of small- and medium-sized blood vessels without lymphadenopathy. Two patients initially presented with fever of unknown origin and persistent lactic acidosis without evidence of tissue hypoxia. Neither patient had an identifiable source of infection and both underwent peripheral blood smear demonstrating leukocytosis with a neutrophilic predominance and thrombocytopenia without evidence of hematologic malignancy. One had previously had a bone marrow biopsy which was unremarkable. Both patients’ condition deteriorated rapidly, progressing to multiorgan failure requiring pressors and mechanical ventilation, which ultimately resulted in cardiopulmonary arrest. At autopsy, each patient demonstrated malignant lymphocytoid cells, staining positive for CD20, localized to the lumina of small- and medium-sized vessels in multiple organs, including the lungs, liver, spleen, and kidneys, among others, allowing for the diagnosis of IVLBCL. IVLBCL is exceedingly rare, which in combination with significant variability in presentation, can make identification and diagnosis challenging. Diagnosis requires biopsy, therefore a high index of suspicion is needed to obtain an adequate tissue sample, whether pre- or postmortem. In the presented cases, both patients exhibited type B lactic acidosis with an unknown etiology that was ultimately determined at autopsy.


Lymphoma, Non-Hodgkin, lactic acidosis


1 Pfleger L, Tappeiner J. On the recognition of systematized endotheliomatosis of the cutaneous blood vessels (reticuloendotheliosis?). Hautarzt. 1959;10:359-63. PMid:14432547.

2 Rajyaguru DJ, Bhaskar C, Borgert AJ, Smith A, Parsons B. Intravascular large B-cell lymphoma in the United States (US): a population-based study using Surveillance, Epidemiology, and End Results program and National Cancer Database. Leuk Lymphoma. 2017;58(9):1-9. PMid:28278725.

3 Murase T, Yamaguchi M, Suzuki R, et al. Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. Blood. 2007;109(2):478-85. PMid:16985183.

4 Ponzoni M, Campo E, Nakamura S. Intravascular large B-cell lymphoma: a chameleon with multiple faces and many masks. Blood. 2018;132(15):1561-7. PMid:30111607.

5 Ponzoni M, Ferreri AJM, Campo E, et al. Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. J Clin Oncol. 2007;25(21):3168-73. PMid:17577023.

6 Desclaux A, Lazaro E, Pinaquy J, Yacoub M, Viallard J. Renal intravascular large B-cell lymphoma: a case report and review of the literature. Intern Med. 2017;56(7):827-33. PMid:28381751.

7 Ronny FMH, Black MA, Arbini AA. Intravascular large B-cell lymphoma with multi-organ failure presenting as a pancreatic mass: a case with atypical presentation and definite diagnosis postmortem. Autops Case Rep. 2017;7(4):30-6. PMid:29259930.

8 Dean RK, Subedi R, Gill D, Nat A. Consideration of alternative causes of lactic acidosis: thiamine deficiency in malignancy. Am J Emerg Med. 2017;35(8):1214.e5-6. PMid:28539246.

9 Foucher CD, Tubben RE. Lactic Acidosis. In: Abai B, editor. StatPearls. Treasure Island: Stat Pearls Publishing; 2021.

10 Liu QS, Harji F, Jones A, Tarnower AC. Type B lactic acidosis: a rare oncological emergency. BMJ Case Rep. 2020;13(3):e233068. PMid:32234853.

11 Estalilla OC, Koo CH, Brynes RK, Medeiros LJ. Intravascular large B-cell lymphoma. A report of five cases initially diagnosed by bone marrow biopsy. Am J Clin Pathol. 1999;112(2):248-55. PMid:10439806.

12 Khalidi HS, Brynes RK, Browne P, Koo CH, Battifora H, Medeiros LJ. Intravascular large B-cell lymphoma: the CD5 antigen is expressed by a subset of cases. Mod Pathol. 1998;11(10):983-8.

13 Orwat DE, Batalis NI. Intravascular large B-cell lymphoma. Arch Pathol Lab Med. 2012;136(3):333-8. PMid:22372911.

14 Matsue K, Abe Y, Narita K, et al. Diagnosis of intravascular large B cell lymphoma: novel insights into clinicopathological features from 42 patients at a single institution over 20 years. Br J Haematol. 2019;187(3):328-36. PMid:31267524.

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